Immune Thrombocytopenia: A Rare Presentation of Pulmonary Sarcoidosis

Thrombocytopenia may be the initial presentation of sarcoidosis, which is a systemic granulomatous disorder. Various pathophysiological mechanisms have been identified. Immune thrombocytopenia often has a severe presentation but may respond favourably to immunosuppressive therapy. There are no guidelines for the treatment of thrombocytopenia in sarcoidosis. However, in emergency situations with major bleeding, it seems reasonable to apply the current guidelines recommended for immune thrombocytopenia. The authors report a case of sarcoidosis presenting with severe thrombocytopenia, petechial rash, and nasal and gingival bleeding.

Small Fiber Neuropathy in Sarcoidosis

Sarcoidosis (SC) is a granulomatous disease of an unknown origin. The most common SC-related neurological complication is a small fiber neuropathy (SFN) that is often considered to be the result of chronic inflammation and remains significantly understudied. This study aimed to identify the clinical and histological correlates of small fiber neuropathy in sarcoidosis patients. The study was performed in 2018–2019 yy and included 50 patients with pulmonary sarcoidosis (n = 25) and healthy subjects (n = 25). For the clinical verification of the SFN, the “Small Fiber Neuropathy Screening List” (SFN-SL) was used. A punch biopsy of the skin was performed followed by enzyme immunoassay analysis with PGP 9.5 antibodies. Up to 60% of the sarcoidosis patients reported the presence of at least one complaint, and it was possible that these complaints were associated with SFN. The most frequent complaints included dysfunctions of the cardiovascular and musculoskeletal systems and the gastrointestinal tract. A negative, statistically significant correlation between the intraepidermal nerve fiber density (IEND) and SFN-SL score was revealed. In patients with pulmonary sarcoidosis, small fiber neuropathy might develop as a result of systemic immune-mediated inflammation. The most common symptoms of this complication were dysautonomia and mild sensory dysfunction.

Efficacy and tolerability of methotrexate at a dose of 15 mg/week and 10 mg/week in patients with pulmonary sarcoidosis

The objective of this study is to conduct a comparative study of the efficacy and safety of methotrexate (MT) at a dose of 10 mg/week and 15 mg/week in patients with pulmonary sarcoidosis having contraindications to GCS therapy. Material and Methods. The study involved 44 patients with stage II pulmonary sarcoidosis (26 females and 18 males aged 24 to 70) with contraindications to the appointment of therapy glucocorticosteroid (GCS). In group 1 (28 patients), methotrexate was prescribed at a dose of 10 mg/week, in group 2 (16 patients), methotrexate was prescribed at a dose of 15 mg/week. The diagnosis and assessment of the dynamics of sarcoidosis were carried out taking into account clinical symptoms based on the results of high-resolution computed tomography and body plethysmography. The significance of differences in indicators was determined using the Student's t-test and Fisher's exact test. The number of cases of clinical treatment without residual changes of a fibrous nature in the lung parenchyma in patients after treatment with methotrexate at a dose of 15 mg/week significantly increased compared to the same indicator in the group of patients after treatment at a dose of 10 mg/week (81.3% and 42.4% respectively, p=0.025). An increase in the therapeutic dose of methotrexate from 10 mg/week to 15 mg/week leads to a decrease in the time to achieve a clinical cure (10.1±0.5 months and 12.8±0.8 months respectively, р˂0.02), indicating an accelerating rate of regression of sarcoidosis. Immunosuppressive therapy of patients with pulmonary sarcoidosis using the drug at doses of 10 and 15 mg/week is characterized by satisfactory tolerability.

T Lymphocyte Maturation Profile in the EBUS-TBNA Lymph Node Depending on the DLCO Parameter in Patients with Pulmonary Sarcoidosis

Sarcoidosis (SA) is a systemic granulomatous disorder of unknown etiology with lung and mediastinal lymph nodes (LNs) as the main location. T lymphocytes play important role in the formation of granulomas in SA, but still little is known about the role of maturation profile in the development of inflammatory changes. The aim of this study was to determine the CD4+ and CD8+ T cells maturation profile in LNs and in peripheral blood (PB) and its relation to disease severity expressed by diffusing capacity of the lung for carbon monoxide (DLCO). 29 patients with newly pulmonary SA were studied. Flow cytometry was used for cells evaluation in EBUS-TBNA samples. We observed lower median proportion of T lymphocytes, CD4+ T and CD8+ T cells in patients with DLCO< 80% than in patients with normal diffusion (DLCO > 80%). Patients with DLCO < 80% had lower median proportion of effector and higher median proportion of central memory CD4+ and CD8+ T cells than patients with DLCO > 80%. We reported for the first time that LNs CD4+ and CD8+ T cells maturation differs depending on the DLCO value in sarcoidosis. Lymphocytes profiles in LNs may reflect the immune status of patients with SA and can be analysed by flow cytometry of EBUS-TBNA samples.